Background: Although arthrogryposis multiplex congenital (AMC) is a rare condition, rapidly progressive scoliosis is common in children with AMC. Only a limited number of studies characterize the nature of these curves, and even fewer describe surgical outcomes. To determine efficacy or rib-based distraction in these patients, we reviewed the outcomes of the use of the Vertical Expandable Prosthetic Titanium Rib (VEPTR) device in what we believe is the first study to report this.
Methods: Search of the Chest Wall Spinal Deformity Study Group database identified 10 children with AMC and early-onset scoliosis who were treated with the VEPTR device at 6 different pediatric health centers. The 7 female and 3 male patients had their initial surgery at an average age of 5 years. Mean follow-up was 4.2 years.
Results: The most common curve was from T5 to L2. After initial VEPTR insertion, the scoliosis decreased from a mean of 67 to 43 degrees (37% correction) and kyphosis from 65 to 48 degrees (29% correction). The mean proximal junctional kyphosis after initial insertion was 33 degrees. At final follow-up, scoliosis and kyphosis were 55 degrees (17% correction) and 62 degrees (8% correction), respectively. Spinal growth during the treatment period showed a mean T1-S1 increase of 4.2 cm or approximately 1 cm/y. In the 62 procedures performed over the course of the study period, 6 complications occurred in 4 patients: 3 infections, 2 rib failures, and 1 implant failure. Six patients had proximal junctional kyphosis of ≥45 degrees at the last follow-up.
Conclusions: In children with AMC, rib-based distraction using the VEPTR is an effective treatment method for controlling scoliosis and kyphosis and maintaining thoracic growth, but proximal junctional kyphosis remains a problem.